TY - JOUR
T1 - Pediatric primary hyperparathyroidism
T2 - Surgical pathology and long-term outcomes in sporadic and familial cases
AU - Szabo Yamashita, Thomas
AU - Gudmundsdottir, Hallbera
AU - Foster, Trenton R.
AU - Lyden, Melanie L.
AU - Dy, Benzon M.
AU - Tebben, Peter J.
AU - McKenzie, Travis
N1 - Funding Information:
Authors report no proprietary or commercial interest in any product mentioned or concept discussed in this article. All research was produced at Mayo Clinic Rochester, MN. This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Publisher Copyright:
© 2022 Elsevier Inc.
PY - 2023/4
Y1 - 2023/4
N2 - Background: Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce. Methods: Single-center retrospective review (1994–2020) of patients ≤21 years undergoing surgery for PHPT. Results: 66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001. Conclusion: Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.
AB - Background: Primary Hyperparathyroidism (PHPT) is rare in pediatric patients. Data regarding surgical outcomes are scarce. Methods: Single-center retrospective review (1994–2020) of patients ≤21 years undergoing surgery for PHPT. Results: 66 patients were identified (61% female, 17 ± 3 years). 71% of patients were symptomatic at diagnosis. 32% of patients had known familial syndromes, most commonly MEN-1. 23% of patients without a known mutation had genetic testing, 22% positive. 56% of the total and 19% of the familial cohort underwent focused exploration. Single gland disease was found in 19% of familial vs 85% of sporadic cases, p < 0.00001. Persistence was 9%, all in the sporadic group, p = 0.11. Recurrence was 15%: 38% in the familial vs 2% in the sporadic groups, p=0.0004. Time to recurrence was 59 months (Q1-38, Q3-95), familial 61 vs 124 months sporadic, p=0.001. Conclusion: Pediatric PHPT is frequently sporadic, although 5% of apparent sporadic cases are secondary to syndromes. Familial cases have higher rates of recurrence, requiring closer follow-up.
KW - Familial hyperparathyroidism
KW - Hypercalcemia
KW - MEN
KW - Pediatric hyperparathyroidism
KW - Primary hyperparathyroidism
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U2 - 10.1016/j.amjsurg.2022.10.018
DO - 10.1016/j.amjsurg.2022.10.018
M3 - Article
C2 - 36270819
AN - SCOPUS:85140082906
SN - 0002-9610
VL - 225
SP - 699
EP - 702
JO - American journal of surgery
JF - American journal of surgery
IS - 4
ER -